CircMYO1B/miR-155 pathway is a common mechanism of stress-induced immunosuppression affecting immune response to three vaccines in chicken.

Stress-induced immunosuppression (SIIS) can weaken the immune response effect of poultry vaccination, and bring huge hidden dangers and economic losses to the poultry industry. However, the detailed molecular mechanisms are still not fully understood. Unveiling the common mechanism of SIIS affecting the immune response to different vaccines is critical for detecting and minimizing the losses caused by SIIS. This study used glucocorticoid dexamethasone (Dex) to simulate SIIS, and three classic avian vaccines (including avian influenza virus (AIV), Newcastle disease virus (NDV), and infectious bursal disease virus (IBDV)) were used to induce immune responses in chicken. Quantitative real-time PCR (qRT-PCR) revealed the expression characteristics and functions of circMYO1B and miR-155 in the processes of SIIS affecting the immune response to the aforementioned avian vaccines, as well as their targeted regulatory relationship. Subsequent bioinformatics analysis predicted FOS, one of the potential target genes of miR-155. The results showed that circMYO1B/miR-155 pathway served as a key common mechanism by which SIIS affected the immune response to the three vaccines. Both heart and proventriculus appeared to be the crucial tissues for this process, with five days post immunization (dpi) emerging as the primary time of interest. Moreover, mitogen-activated protein kinase (MAPK) signaling system played a key role in modulating the immune response subsequent to SIIS administration. Our findings provide new insights into the immune function of competitive endogenous RNA (ceRNA), which have important function in the detection and treatment of SIIS affecting vaccine immunity.

Stress-Induced Immunosuppression Inhibits Regional Immune Responses in Chicken Adipose Tissue Partially through Suppressing T Cells by Up-Regulating Steroid Metabolism.

Lipid metabolism plays an important role in maintaining lipid homeostasis and regulating immune functions. However, the regulations and mechanisms of lipid metabolism on the regional immune function of avian adipose tissue (AT) have not been reported. In this study, qRT-PCR was used to investigate the changes and relationships of different lipid metabolism pathways in chicken AT during stress-induced immunosuppression (SIIS) inhibiting immune response to Newcastle disease virus vaccine, then the miRNA regulation patterns of 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) gene and its potential applications were further identified. The results showed that AT actively responded to SIIS, and ATGL, CPT1A and HMGCR were all the key genes involved in the processes of SIIS inhibiting the immune responses. SIIS significantly inhibited the natural and specific immune phases of the primary immune response and the initiation phase of the secondary immune response in AT by suppressing T cells by up-regulating steroid anabolism. Moreover, steroid metabolism could play dual roles in regulating the regional immune functions of AT. The miR-29a/c-3p-HMGCR network was a potential regulation mechanism of steroid metabolism in AT, and serum circulating miR-29a/c-3p had the potential as molecular markers. The study can provide valuable references for an in-depth investigation of the regional immune functions regulated by lipid metabolism in AT.

O-GlcNAcylation of melanophilin enhances radiation resistance in glioblastoma via suppressing TRIM21 mediated ubiquitination.

The molecular mechanism of glioblastoma (GBM) radiation resistance remains poorly understood. The aim of this study was to elucidate the potential role of Melanophilin (MLPH) O-GlcNAcylation and the specific mechanism through which it regulates GBM radiotherapy resistance. We found that MLPH was significantly upregulated in recurrent GBM tumor tissues after ionizing radiation (IR). MLPH induced radiotherapy resistance in GBM cells and xenotransplanted human tumors through regulating the NF-κB pathway. MLPH was O-GlcNAcylated at the conserved serine 510, and radiation-resistant GBM cells showed higher levels of O-GlcNAcylation of MLPH. O-GlcNAcylation of MLPH protected its protein stability and tripartite motif containing 21(TRIM21) was identified as an E3 ubiquitin ligase promoting MLPH degradation whose interaction with MLPH was affected by O-GlcNAcylation. Our data demonstrate that MLPH exerts regulatory functions in GBM radiation resistance by promoting the NF-κB signaling pathway and that O-GlcNAcylation of MLPH both stabilizes and protects it from TRIM21-mediated ubiquitination. These results identify a potential mechanism of GBM radiation resistance and suggest a potential therapeutic strategy for GBM treatment.

Structural elucidation of a non-starch polysaccharides from Lilii Bulbus and its protective effects against corticosterone-induced neurotoxicity in PC12 cells.

Lilii Bulbus is a folk medicine for both culinary and medicinal purpose. In traditional medicine theory, Lilii Bulbus is usually used as an complementary therapy for nourishing the heart and lung, clearing heat in the treatment of mental instability and depression. In this study, NLPS-1a (Mw = 2610 Da, DP = 16), a water-soluble non-starch Lilii Bulbus polysaccharides, was isolated and purified. Structural analysis showed that NLPS-1a mainly contained Man and Glc with a molar ratio of 11.137 and 9.427. The glycosidic linkages of NLPS-1a were 1,3-Manp (59.93%), 1,2-Glcp (37.93%), T-Glcp (1.21%) and T-Manp (0.93%), indicating the highly-linear structures. In addition, NLPS-1a could significantly repair the injury of PC12 cells induced by corticosterone (CORT), reduce Lactate dehydrogenase (LDH) leakage and decrease the cell apoptosis in a dose-dependent manner. Above all, the results indicated that NLPS-1a had protective effects against CORT-induced neurotoxicity in PC12 cells, and might be a natural antidepressant, which enriched the study of the metabolic mechanism between herbal polysaccharides and antidepressant.

Structural changes of polysaccharides from Astragulus after honey processing and their bioactivities on human gut microbiota.

BACKGROUND: Astragali Radix (also known as Astragulus) is a traditional medicinal and edible homologous plant for tonifying Qi. Honey-processed Astragalus is a dosage form of Astragali Radix processed with honey, which exhibited better efficacy of tonifying Qi than the raw product. Polysaccharides are their main active components. RESULTS: APS2a and HAPS2a were initially isolated from Astragulus and honey-processed Astragulus. Both of them are highly branched acidic heteropolysaccharides containing ɑ-configuration and ß-configuration glycosidic bonds. The molecular weight and the molecular dimension of HAPS2a decreased and the GalA contained in APS2a was converted to Gal in HAPS2a. The α-configuration galactose residue 1,3,4-α-Galp in the backbone of APS2a was converted to the corresponding ß-configuration galactose residue 1,3,4-ß-Galp in the backbone of HAPS2a and the uronic acid residue T-α-GalpA in the sidechain of APS2a was converted to the corresponding neutral residue T-α-Galp in the side chain of HAPS2a. Bioactivity results showed that HAPS2a had better probiotic effects on Bacteroides ovatus, Bacteroides thetaiotaomicron, Bifidobacterium longum and Lactobacillus rhamnosus strains than APS2a. After degradation, the molecular weights of HAPS2a and APS2a decreased with the changes in their monosaccharide composition. The contents of total short-chain fatty acids (SCFAs) and other organic acids in HAPS2a group were higher than APS2a group. CONCLUSIONS: Two novel high-molecular-weight polysaccharides named APS2a and HAPS2a had different probiotic activities in vitro, which might be due to their structural differences before and after honey processing. Both of them might be possibly used as an immunopotentiator in healthy foods or dietary supplement. © 2023 Society of Chemical Industry.

Regional immunity of chicken adipose tissue responds to secondary immunity induced by Newcastle disease vaccine via promoting immune activation and weakening lipid metabolism.

Adipose tissue (AT) is considered as a regional immune organ and plays an important role in the anti-infection immune response. However, the function and mechanism of chicken AT in response to secondary immune response remain poorly understood. Here, we used mRNA and microRNA (miRNA) sequencing technology to survey the transcriptomic landscape of chicken abdominal adipose tissue (AAT) during the first and second immunization with Newcastle disease virus (NDV) vaccine, and carried out bioinformatics analysis, such as Gene Ontology (GO) terms and Kyoto Encyclopedia of Genes and Genomes (KEGG) functional enrichment analysis, protein-protein interaction (PPI) analysis, and miRNA-mRNA integrated analysis. The results indicated that chicken AAT actively responded to the secondary immune response. DNA replication and cytoskeleton regulation as the regulatory functions of immune activation changed significantly, and weakened lipid metabolism was an effective strategy for the secondary immunity. Mechanically, the regulatory network between the differentially expressed miRNAs (DEMs) and their targeted differentially expressed genes (DEGs), such as miR-206/miR-499-5p-nuclear receptor subfamily 4 group A member 3 (NR4A3)/methylsterol monooxygenase 1 (MSMO1) pathway, was one of the potential key mechanisms by which AAT responded to the secondary immune response. In conclusion, regional immunity of chicken AT responds to secondary immunity by promoting immune activation and weakening lipid metabolism, and this study can instruct future research on antiviral strategy.

Circular RNA circAKIRIN2 participates in the process of stress-induced immunosuppression affecting immune response to infectious bursal disease virus vaccine in chicken.

At present, stress-induced immunosuppression is still a hidden threat that leads to immunization failure and outbreaks of poultry diseases, and causes huge economic losses to the modern poultry industry. However, the molecular mechanisms of stress-induced immunosuppression affecting viral vaccine immunity are still poorly understood. Here, we identified circAKIRIN2 as a conserved circular transcript in chicken, and explored its expression patterns in different immune states by quantitative real-time PCR (qRT-PCR), then conducted bioinformatics analysis. The results showed that circAKIRIN2 actively participated in the process of stress-induced immunosuppression affecting the immune response to infectious bursal disease virus (IBDV) vaccine. The key time points for circAKIRIN2 involving in the process were 2 day post immunization (dpi), 5 dpi, and 28 dpi, especially at the acquired immune stage. The important tissues that responded to the process included the heart, liver, and lung, all of which changed significantly. In addition, circAKIRIN2 as a competing endogenous RNA (ceRNA) sponging zinc finger and BTB domain containing 20 (ZBTB20) was a potential molecular mechanism for regulating immune functions in the process. In conclusion, circAKIRIN2 is a key regulatory factor for stress-induced immunosuppression affecting the IBDV vaccine immune response, and this study can provide a new perspective for exploring the molecular regulatory mechanisms of stress-induced immunosuppression affecting immune response.

Stress-induced immunosuppression affecting immune response to Newcastle disease virus vaccine through "miR-155-CTLA-4" pathway in chickens.

MiR-155 and CTLA-4 are important factors involved in the regulation of immune function. However, there is no report about their involvement in function regulation of stress-induced immunosuppression affecting immune response. In this study, the chicken model of stress-induced immunosuppression affecting immune response (simulation with dexamethasone and immunization with Newcastle disease virus (NDV) attenuated vaccine) was established, then the expression characteristics of miR-155 and CTLA-4 gene were analyzed at several key time points during the processes of stress-induced immunosuppression affecting NDV vaccine immune response at serum and tissue levels. The results showed that miR-155 and CTLA-4 were the key factors involved in stress-induced immunosuppression and NDV immune response, whose functions involved in the regulation of immune function were different in different tissues and time points, and 2 day post immunization (dpi), 5dpi and 21dpi were the possible key regulatory time points. CTLA-4, the target gene of miR-155, had significant game regulation relationships between them in various tissues, such as bursa of Fabricius, thymus and liver, indicating that miR-155-CTLA-4 pathway was one of the main mechanisms of their involvement in the regulations of stress-induced immunosuppression affecting NDV immune response. This study can lay the foundation for in-depth exploration of miR-155-CTLA-4 pathway involved in the regulation of immune function.

Fetuin-A alleviates neuroinflammation against traumatic brain injury-induced microglial necroptosis by regulating Nrf-2/HO-1 pathway.

BACKGROUND: The microglia-mediated inflammatory response is a vital mechanism of secondary damage following traumatic brain injury (TBI), but the underlying mechanism of microglial activation is unclear. METHODS: Controlled cortical impact (CCI) was induced in adult male C57BL/6J mice, and glutamate was used to construct a classical in vitro injury model in the primary microglia. Microglial activation was determined by western blot and immunostaining. The inflammatory factors were measured by enzyme-linked immunosorbent assay. The oxidative stress marker and mitochondrial reactive oxygen species (ROS) were measured by immunoblotting and MitoSox Red staining. Transmission electron microscopy was used to observe the typical morphology of necroptotic cells. RESULTS: Our quantitative proteomics identified 2499 proteins; 157 were significantly differentially expressed in brain tissue between the 6 h after CCI (CCI6h) group and sham group, and 109 were significantly differentially expressed between the CCI24h and sham groups. Moreover, compared with the sham group, the terms "acute-phase response", "inflammation", and "protein binding" were significantly enriched in CCI groups. Fetuin-A, a liver-secreted acute-phase glycoprotein, was involved in these biological processes. Using an experimental TBI model, we found that the Fetuin-A level peaked at 6 h and then decreased gradually. Importantly, we showed that administration of Fetuin-A reduced the cortical lesion volume and edema area and inhibited the inflammatory response, which was associated with suppressing microglial necroptosis, thus decreasing microglial activation. Furthermore, administration of Fetuin-A attenuated mitochondrial oxidative stress in glutamate-treated microglial cells, which is a critical mechanism of necroptosis suppression. In addition, we demonstrated that Fetuin-A treatment promoted translocation of nuclear factor erythroid 2-related factor 2 (Nrf-2) from the cytoplasm to the nucleus in vivo; however, the Nrf-2 inhibitor ML385 and si-heme oxygenase-1 (si-HO-1) disrupted the regulation of oxidative stress by Fetuin-A and induced increased ROS levels and necroptosis in glutamate-treated microglial cells. Fetuin-A also protected neurons from adverse factors in vivo and in vitro. CONCLUSIONS: Our results demonstrated that Fetuin-A activated Nrf-2/HO-1, suppressed oxidative stress and necroptosis levels, and thereby attenuates the abnormal inflammatory response following TBI. The findings suggest a potential therapeutic strategy for TBI treatment.

Circulating miR-155, a potential regulator of immune responses to different vaccines in chicken.

Vaccination is one of effective means to prevent viral infectious diseases in poultry. However, the functions of circulating miRNAs in immune response remain unknown. In this study, miR-155, a key factor in the regulation of immune function, was selected to study its expression, potential function and mechanism in the 12-day-old chicken immune responses to three vaccines (avian influenza virus inactivated vaccine, Newcastle disease virus attenuated vaccine and infectious bursal disease virus attenuated vaccine), respectively. The experiment aimed to explore the relationships between the expression levels of serum circulating miR-155 and immune responses. The results showed that the expression levels of serum circulating miR-155 were significantly different during the three immune responses, but had similarities at several time points post inoculation. 2 day post inoculation (dpi), 5dpi, and 21dpi were the possible common key time points of the three immune responses. Moreover, spleen (2dpi), bursa of Fabricius and cecal tonsil (5dpi), and liver (21dpi) were the possible key tissues associated with the differential expression levels of serum circulating miR-155. Bioinformatics analysis showed that several key target genes (such as KRAS, RAP1B, and RPS6KA3) of miR-155 possibly played a key role in immune function regulation through MAPK and mTOR signaling pathways. The study can lay the foundation for further studying the function and application of circulating miR-155 in chicken immune responses.

Stress-Induced Immunosuppression Affects Immune Response to Newcastle Disease Virus Vaccine via Circulating miRNAs.

Studies have shown that circulating microRNAs (miRNAs) are important players in the immune response and stress-induced immunosuppression. However, the function and mechanism of stress-induced immunosuppression affecting the immune response to the Newcastle disease virus (NDV) vaccine remain largely unknown. This study analyzed the changes of 15 NDV-related circulating miRNAs at different immune stages by qRT-PCR, aiming to explore the key timepoints, potential biomarkers, and mechanisms for the functional regulation of candidate circulating miRNAs under immunosuppressed conditions. The results showed that stress-induced immunosuppression induced differential expressions of the candidate circulating miRNAs, especially at 2 days post immunization (dpi), 14 dpi, and 28 dpi. In addition, stress-induced immunosuppression significantly affected the immune response to NDV vaccine, which was manifested by significant changes in candidate circulating miRNAs at 2 dpi, 5 dpi, and 21 dpi. The featured expressions of candidate circulating miRNAs indicated their potential application as biomarkers in immunity and immunosuppression. Bioinformatics analysis revealed that the candidate circulating miRNAs possibly regulated immune function through key targeted genes, such as Mg2+/Mn2+-dependent 1A (PPM1A) and Nemo-like kinase (NLK), in the MAPK signaling pathway. This study provides a theoretical reference for studying the function and mechanism of circulating miRNAs in immune regulation.

Stress-induced immunosuppression affecting avian influenza virus vaccine immune response through miR-20a-5p/NR4A3 pathway in chicken.

Stress-induced immunosuppression is one of the most common hazards in poultry intensive production, which often leads to vaccination failure and severe economic losses. At present, there is no report about the function and mechanism of circulating miRNA on stress-induced immunosuppression affecting immune response. In this study, the changes of circulating miR-20a-5p under stress-induced immunosuppressive condition were analyzed by qRT-PCR, and the key time points, tissues and mechanisms for functional regulation of miR-20a-5p in the process of stress-induced immunosuppression affecting avian influenza virus (AIV) vaccine immune response were identified. The results showed that stress-induced immunosuppression down-regulated miR-20a-5p and further affected AIV vaccine immune response, in which 5 day post immunization (dpi) was a key time point, and the heart, lung, and proventriculus were the important tissues. The game relationship analysis between miR-20a-5p and its target nuclear receptor subfamily 4 group A member 3 (NR4A3) gene showed that "miR-20a-5p/NR4A3" pathway was the potential key mechanism of this process, especially for heart and lung. This study provides insights into the molecular mechanisms of stress-induced immunosuppression affecting immune response.

An important link between the gut microbiota and the circadian rhythm: imply for treatments of circadian rhythm sleep disorder.

Currently, gut microbiota living in the gastrointestinal tract, plays an important role in regulating host's sleep and circadian rhythms. As a tool, gut microbiota has great potential for treating circadian disturbance and circadian insomnia. However, the relationship between gut microbiota and circadian rhythms is still unclear, and the mechanism of action has still been the focus of microbiome research. Therefore, this article summarizes the current evidences associating gut microbiota with factors that impact host circadian rhythms neurology sleep disorder. Moreover, we discuss the changes to these systems in sleep disorder and the potential mechanism of intestinal microbiota in regulating circadian rhythms neurology sleep disorder via microbial metabolites. Meanwhile, based on the role of intestinal flora, it is provided a novel insight into circadian related insomnia and will be benefit the dietary treatment of circadian disturbance and the circadian related insomnia.

Stress-induced immunosuppression increases levels of certain circulating miRNAs and affects the immune response to an infectious bursal disease virus vaccine in chickens.

Stress-induced immunosuppression can affect the immune effect of vaccine. However, the mechanism of stress-induced immunosuppression affecting immune response to infectious bursal disease virus (IBDV) vaccine in chicken is still unclear. In this study, thirteen IBDV related circulating miRNAs were selected to study their expressions, possible functions and mechanisms in dexamethasone (Dex)-induced immunosuppressed chicken vaccinated with IBDV attenuated vaccine. The experiment aimed to explore the relationship between the expressions of IBDV related circulating miRNAs and stress-induced immunosuppression. The quantitative real-time PCR (qRT-PCR) results showed that Dex-induced immunosuppression could induce the differential expressions of the candidate serum circulating miRNAs, especially on the 2nd, 5th, 7th and 28th day after dexamethasone treatment. Dex-induced immunosuppression could affect the immune response to the IBDV vaccine, which was possibly achieved by partially regulating the differential expressions of the IBDV related circulating miRNAs. Bioinformatics analysis showed that the candidate miRNAs could regulate the immune function mainly through targeting genes (such as CREB1 and MAPK1) in TGF-ß and MAPK signaling pathways. This study can provide a preliminary reference for further studying the function and mechanism of circulating miRNAs in immune regulation.

Screen-Printed High-Performance Flexible Electrothermal Films Based on Three-Dimensional Intercalation Graphene Nanosheets/MWCNT/Carbon Black Composite.

A liquid-phase stripping method was used to strip the graphite under the action of mechanical shear force to prepare graphene nanosheets (GNSs) on a large scale. Given the multicomponent composite conductive particles formed by GNSs with acid-treated MWCNTs (f-MWCNTs) and carbon black (CB), the three-dimensional (3D) intercalation electrothermal composite of GNSs/MWCNTs/CB with excellent conductivity and mechanical properties was prepared with water-based acrylic resin as a connector. Carbon particles (16.97 wt %) are found in the composite and the sheet resistance (Rs) is only 4 Ω sq-1 as f-MWCNTs and CB intercalations form a more stable 3D conducting medium between the GNSs. The flexible electrothermal film (2.5 cm × 2.5 cm) printed with the 3D intercalation GNSs/MWCNTs/CB composite had a saturation temperature (Ts) of 175 °C with an input of 3 V and lower power consumption (249.87 cm2 W-1). It only takes 10 s to reach Ts and the electrical performance is still intact under the pressure of 1 × 105 kPa. After being bent 2500 times (bending radius is 5 mm), the electrothermal performance of the flexible electrothermal film remained stable.

A dual cancer-specific recombinant adenovirus suppresses the growth of liver cancer cells in vivo and in vitro.

Oncolytic virus therapy is emerging as important means in cancer treatment. In a previous study, we constructed a dual cancer-specific antitumor recombinant adenovirus, designating it Ad-apoptin-hTERTp-E1a (Ad-VT). This study aimed to investigate the anticancer potential of recombinant adenovirus Ad-apoptin-hTERTp-E1a (Ad-VT) in liver cancer. Crystal Violet staining and CCK-8 assays were used to analyse the inhibitory effect of recombinant adenovirus on human hepatoma cell line QGY-7703 and SMMC-7721. Ad-VT had a significant tumour killing inhibitory effect on QGY-7703 and SMMC-7721 cells that was both dose and a time dependent. Ad-VT-induced apoptosis of QGY-7703 cells was detected using Hoechst, Annexin V, and JC-1 staining, as well as western blotting. Recombinant adenovirus had a strong apoptosis-inducing effect on QGY-7703 cells, and killed QGY-7703 cells mainly through the mitochondrial apoptotic pathway. QGY-7703 cells invasion were detected using cell-scratch and Transwell assays. Recombinant adenovirus could significantly inhibit the invasion of QGY-7703 cells over a short period of time. The pGL4.51 plasmid was used to transfect QGY-7703 cells to construct tumour cells stably expressing luciferase (QGY-7703-LUC). The tumour inhibition effect of Ad-VT in vivo was subsequently confirmed by establishing a tumour-bearing nude mouse model. Ad-VT could effectively inhibit tumour growth and prolong survival of the mice. Recombinant adenovirus Ad-VT has the characteristics of tumour-specific replication and specific tumour killing, and could inhibit the growth of liver cancer QGY-7703 cells and promote their apoptosis.

The key local segments of human body for personalized heating and cooling.

Thermal characteristics of local body parts of a human subject are markedly different in cold or hot environments. Some body segments are known to be much more susceptible to heat loss than the others, thus strongly influencing the overall thermal sensation of a subject. If these body parts can be effectively cooled in a hot environment or warmed in a cold environment using personal environmental control systems, thermal comfort of human occupants can be achieved at the minimum cost of energy without heavily relying on centralized heating, ventilation, and air conditioning systems. With an objective to understand the influence of local thermal sensation on the subjects' overall thermal comfort perception, experiments in the two sets of climate chambers were carried out simulating summer and winter conditions, respectively. A total of 24 subjects (12 females and 12 males) were recruited for this study, and their local skin temperature, conductive heat flux, and thermal sensations were recorded during the experiments. The local thermal characteristics of the subjects were compared between the 'neutral' and 'hot' conditions to identify predominant body segments in the summer scenario. Moreover, the comparison was also made between the 'neutral' and 'cold' conditions to derive predominant body segments in the winter scenario. The analysis of the results indicated that leg, thigh, and back are the key segments desirable for local cooling; whilst leg, thigh, back, and upper arm are the crucial segments for local heating. The findings can have important implications for the design of low-energy cost-effective personal heating/cooling devices. Finally, the results identified the conductive heat flux of skin as a useful physiological parameter in examining human thermal sensation.

Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment.

Angiosarcoma is an aggressive mesenchymal sarcoma of endothelial cell origin with high mortality. Its occurrence in the small intestine is exceedingly low. In addition to the rarity of small intestine angiosarcoma, the nonspecific early clinical symptoms obscure the suspicion of such tumors and thereby delay the diagnosis. In a hope to improve the knowledge of this rare but fatal neoplasm, we report one case of angiosarcoma of duodenum and jejunum in a 73-year-old man. Furthermore, we summarize and analyze the common clinical features, tumor markers, treatment, and survival of previous reported cases of this malignancy. Small bowel angiosarcoma occurs more often in men than women (1.6:1). The median age at diagnosis is 68.5 years. The overall median survival time is 150 days; the median survival time in female (300 days) is longer than that of male patients (120 days). Von Willebrand factor (vWF), CD31, CD34, vimentin, and Ulex europaeus agglutinin 1 appear to be the most useful markers for the diagnosis. The majority of the patients underwent surgical resection alone or surgery with subsequent chemotherapy. The patients treated with surgery plus chemotherapy survive longer than those underwent surgical resection only (median 420 days, n = 7 vs. 96.5 days, n = 26, respectively; P = 0.0275). Further studies of more cases are needed for a better understanding of this rare entity, as well as the development of effective strategies for prevention, early diagnosis, and treatment.